Microarray expression profiling in zebrafish rnaset2 mutant
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https://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE138493
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RNASET2-deficient leukoencephalophathy is a severe leukodystrophy affecting children with psychomotor impairements in their forst year of life. We generated the first zebrafish model for a human leukodystrophy by targetting the ortholog rnaset2 gene using mutagenesis. This zebrafish mutant recapitulated the human clinical manisfestations and developed white matter defects detectable by MRI. Additionally, this zebrafish mutant identified this disease as a lysosomal storage disorders, with RNA accumulating in neurons. To understand how accumulation of RNA in neurons trigger white matter lesions, we undertook an unbiased approach and perfored microarray analysis. We identified differentially expressed genes in mutants and the immune system as a key pathway disregulated in the zebrafish rnaset2 mutant. Gene expression profiles of rnaset2 zebrafish compared to WT sibling of whole embryos at 28hour post fertilisation, 3days post fertilisation and brain of 1 year old adults ***Please note that the original/unedited raw data files are unavailable and thus, not provided.
创建时间:
2021-07-07



